Whipple's disease: a report of 22 patients

Twenty-two patients with Whipple's disease are reviewed (21 male, 1 female; mean age 49.3 years). All but one were diagnosed by small intestinal biopsy. The most frequent clinical symptoms at diagnosis were weight loss (14/21 patients), diarrhea (13/21), arthralgias (13/21), cramping abdominal pain (11/21), and skin pigmentation (8/21), which anteceded the diagnosis by from 6 months to 10 years (median 3.5 years). All but three patients were treated successfully with tetracycline given for at least 2 years. Follow-up was done for a median of 7.7 years (range 1-15 years). Two of 17 patients who were followed for 2 years relapsed, as did 2/11 followed for at least 5 years. One patient relapsed twice. Each relapse could be treated with success. In no case did relapse occur in the central nervous system. Thus, tetracycline appears to be effective in the treatment of Whipple's disease if given for at least 2 years. In two further patients with confirmed central nervous system involvement at diagnosis, who were treated with ampicillin plus chloramphenicol, characteristic SPC cells disappeared from the cerebrospinal fluid. In addition, the recently recognized problems in the differential diagnosis of Whipple's disease and atypical mycobacterial infection in AIDS patients are discussed.